Non-specific interstitial pneumonia

Non-specific interstitial pneumonia
Synonyms	Non-specific interstitial pneumonitis
Pronounce	N/A
Specialty	N/A
Symptoms	Cough, difficulty breathing, fatigue
Complications	Respiratory failure, pulmonary hypertension
Onset	Insidious onset, gradual worsening of symptoms
Duration	Chronic, progressive
Types	Cellular pattern, fibrosing pattern
Causes	Possible autoimmune mechanism, associated with undifferentiated connective tissue disease
Risks	Previous lung disease such as COPD or emphysema, autoimmune disorders
Diagnosis	Lung biopsy (VATS or bronchoscopy), histopathological examination of tissue
Differential diagnosis	Usual interstitial pneumonia, other types of idiopathic interstitial pneumonia
Prevention	No known prevention
Treatment	Prednisone, Imuran, Cellcept, antibiotics like Levaquin for pneumonia, oxygen therapy
Medication	Immunosuppressive drugs, antibiotics
Prognosis	Cellular pattern: 100% 5-year survival rate; Fibrosing pattern: 86-92% 5-year survival rate
Frequency	Rare
Deaths	Can result in respiratory failure if untreated

Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia characterized by inflammation and fibrosis of the lung interstitium.

Symptoms

The main symptoms of NSIP include:

• Cough
• Difficulty breathing
• Fatigue

These symptoms typically develop gradually and worsen over time.

Causes

While the exact cause of NSIP is not well understood, it has been suggested that the condition may have an autoimmune basis. It has been identified as a potential complication of undifferentiated connective tissue disease, though more research is needed to fully understand the mechanism. NSIP may also be associated with other lung diseases such as COPD and emphysema, as well as other autoimmune disorders.

Diagnosis

A definitive diagnosis of NSIP can only be made through a lung biopsy, typically performed via Video-assisted thoracoscopic surgery (VATS) by a cardio-thoracic surgeon. While a bronchoscopy may be attempted by some pulmonologists, it often fails to provide an accurate diagnosis.

Upon examination of the lung tissue, two histopathological patterns of NSIP can be identified:

• Cellular pattern: Characterized by chronic inflammation with minimal fibrosis. This pattern is associated with a better prognosis.
• Fibrosing pattern: Characterized by interstitial fibrosis, with varying levels of inflammation. This pattern tends to have a worse prognosis.

Both patterns lack the prominent fibroblastic foci that are found in other forms of idiopathic interstitial pneumonia.

Treatment

Treatment for NSIP typically involves:

• Immunosuppressive therapy: Drugs like Prednisone, Imuran, and Cellcept are commonly used to reduce inflammation and prevent further lung damage.
• Antibiotics: For active infections, antibiotics such as Levaquin are commonly used to treat pneumonia.
• Oxygen therapy: Nearly all patients with NSIP will require oxygen supplementation to manage respiratory symptoms.

Prognosis

The prognosis for NSIP varies based on the histopathological pattern:

• Cellular pattern: The five-year survival rate is around 100%, with better outcomes compared to the fibrosing pattern.
• Fibrosing pattern: The five-year survival rate is lower, ranging from 86% to 92%.

Overall, patients with NSIP tend to have a better prognosis than those with usual interstitial pneumonia (UIP).

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